TABLE 2.
Descriptive Features of the 500 Gut Malrotated Patients According to Status of Gut Anatomy at Time of Referral
| Variable | Midgut-loss | Intact Midgut | P Value |
| Number of patients (%) | 204 (41) | 296 (59) | NA |
| Geographic distribution (%) | 0.322 | ||
| National | 190 (93) | 279 (94) | |
| International | 14 (7) | 17 (6) | |
| Sex (Female / Male) | 90 / 114 | 186 / 110 | <0.0001 |
| Race (White / African-American) | 180 / 24 | 268 / 28 | 0.002 |
| Age at time of diagnosis (median [IQR], yr) | 1 [0–8] | 29 [10–52] | <0.0001 |
| ≤1 | 131 (64) | 42 (14) | |
| >1 to < 18 | 43 (21) | 58 (20) | |
| 18 to ≤ 40 | 24 (12) | 96 (32) | |
| >40 to ≤60 | 6 (3) | 55 (19) | |
| ≥60 | 0 (0) | 45 (15) | |
| Prematurity (%) | 96 (47) | 30 (10) | <0.0001 |
| Associated abdominal congenital anomalies (%) | 143 (70) | 73 (25) | <0.0001 |
| Gastroschisis | 67 (47) | 10 (14) | <0.0001 |
| Intestinal atresia | 52 (36) | 13 (18) | <0.0001 |
| Gastroschisis + intestinal atresia | 15 (11) | 0 (0) | <0.0001 |
| Accessory gut organs | 9 (6) | 50 (68) | <0.0001 |
| Time between symptoms and diagnosis (yr) | |||
| 0–5 | 172 (84) | 169 (57) | <0.0001 |
| >5 | 32 (16) | 127 (43) | |
| History of volvulus (%) | 198 (97) | 56 (19) | <0.0001 |
| History of Ladd's procedure (%) | 50 (25) | 142 (48) | <0.0001 |
| Open | 45 (90) | 98 (69) | |
| Laparoscopic | 5 (10) | 44 (31) | |
| Prior abdominal surgery | |||
| Number of patients (%) | 200 (98) | 241 (81) | <0.0001 |
| Number of procedures (median [IQR]) | 3 [2–5] | 2 [1–4] | <0.0001 |
| Prior organ/cell transplant (%) | 6 (3) | 20 (7) | 0.025 |
| Gut dysmotility (%) | 16 (8) | 40 (14) | <0.0001 |
| Connective tissue / autoimmune (%) | 5 (2) | 20 (7) | <0.0001 |
| Genetic syndromes (%) | 15 (7) | 36 (12) | 0.104 |
| Thrombophilia (%) | 19 (10) | 6 (2) | 0.228 |
| Total parenteral nutrition (%) | 199 (98) | 29 (10) | <0.0001 |
| Surgical management (%) | 189 (93) | 80 (27) | <0.0001 |
| Gut transplantation (GT) | 174 (92)∗ | 0 (0) | |
| Autologous gut reconstruction (AGR) | 15 (8) | 0 (0) | |
| Gut malrotation correction (GMC) surgery | 0 (0) | 80 (100) | |
| Overall survival (%)† | 123 (60) | 260 (88) | <0.0001 |
NA indicates non-applicable.
∗
Sixteen patients failed prior autologous gut reconstruction.
†
as of February 15, 2021.